Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection

Manuel Ramos-Casals, Luca L.A. La Civita, Salvatore De Vita, Roser Solans, Mario Luppi, Francisco Medina, Paola Caramaschi, Patrizia Fadda, Ginevra De Marchi, Armando Lopez-Guillermo, Josep Font, Pilar Brito-Zerón, Veronique Loustaud-Ratti, Margit Zeher, Peter Szodoray, Jose Angel Bosch, Albert Selva-O'Callaghan, Miquel Vilardell, Eric Toussirot, Daniel WendlingJose Rosas, Juan Manuel Anaya, Xavier Forns, Jose Sanchez-Tapias, Luis Javier Jara

Resultado de la investigación: Contribución a una revistaArtículorevisión exhaustiva

48 Citas (Scopus)

Resumen

Objective. To characterize the clinical and immunologic patterns of expression, response to therapy, and outcome of patients with Sjögren's syndrome (SS) and associated hepatitis C virus (HCV) infection who developed B cell lymphoma. Methods. Various international reference centers constituted a multicenter study group with the purpose of creating a registry of patients with SS-HCV who developed B cell lymphoma. A protocol form was used to record the main characteristics of SS, chronic HCV infection, and B cell lymphoma. Results. Twenty-five patients with SS-HCV with B cell lymphoma were included in the registry. There were 22 (88%) women and 3 (12%) men (mean age 55, 58, and 61 years at SS, HCV infection, and lymphoma diagnosis, respectively). The main extraglandular SS manifestations were cutaneous vasculitis in 15 (60%) patients and peripheral neuropathy in 12 (48%); the main immunologic features were positive rheumatoid factor (RF) in 24 (96%) and type II cryoglobulins in 20 (80%). The main histologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma in 11 (44%) patients, diffuse large B cell lymphoma in 6 (24%), and follicular center cell lymphoma in 6 (24%). Fifteen (60%) patients had an extranodal primary location, most frequently in the parotid gland (5 patients), liver (4 patients), and stomach (4 patients). Twelve (52%) of 23 patients died after a median followup from the time of lymphoma diagnosis of 4 years, with lymphoma progression being the most frequent cause of death. Survival differed significantly between the main types of B cell lymphoma. Conclusion. Patients with SS-HCV and B cell lymphoma are clinically characterized by a high frequency of parotid enlargement and vasculitis, an immunologic pattern overwhelmingly dominated by the presence of RF and mixed type II cryoglobulins, a predominance of MALT lymphomas, and an elevated frequency of primary extranodal involvement in organs in which HCV replicates (exocrine glands, liver, and stomach).

Idioma originalInglés estadounidense
Páginas (desde-hasta)161-170
Número de páginas10
PublicaciónArthritis Care and Research
Volumen57
N.º1
DOI
EstadoPublicada - feb 15 2007
Publicado de forma externa

All Science Journal Classification (ASJC) codes

  • Reumatología

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